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Volume 14
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Review

Recent Advances in the Therapeutic Management of Advanced Systemic Mastocytosis

by
Scott Veitch
* and
Deepti H. Radia
*
Department of Haematology, Guy’s and St Thomas’ NHS Foundation Trust, London SE1 7EH, UK
*
Authors to whom correspondence should be addressed.
Diagnostics 2024, 14(1), 80; https://doi.org/10.3390/diagnostics14010080
Submission received: 29 November 2023 / Revised: 22 December 2023 / Accepted: 26 December 2023 / Published: 29 December 2023
(This article belongs to the Special Issue Current Challenges in Diagnosis and Management of Mast Cell Disorders)
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Abstract

Advanced systemic mastocytosis (AdvSM) is a rare haematological neoplasm characterised by the accumulation of neoplastic mast cells (MCs) in various organs, resulting in organ dysfunction and reduced life expectancy. The subtypes include aggressive SM (ASM), SM with an associated haematological neoplasm (SM-AHN) and mast cell leukaemia (MCL). The gain of function KIT D816V mutation is present in most cases. The availability of tyrosine kinase inhibitors (TKIs) has revolutionised the treatment landscape for patients with this life-limiting disease. Patients are now able to achieve molecular remission, improved quality of life and improved overall survival. This review focuses on the targeted therapies currently available in clinical practice and within the clinical trial setting for AdvSM. This review also highlights possible future therapeutic targets and discusses therapeutic strategies for this multimutated and clinically heterogeneous disease.
Keywords: advanced systemic mastocytosis; tyrosine kinase inhibitors; KIT inhibitors; midostaurin; avapritinib; bezuclastinib advanced systemic mastocytosis; tyrosine kinase inhibitors; KIT inhibitors; midostaurin; avapritinib; bezuclastinib

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MDPI and ACS Style

Veitch, S.; Radia, D.H. Recent Advances in the Therapeutic Management of Advanced Systemic Mastocytosis. Diagnostics 2024, 14, 80. https://doi.org/10.3390/diagnostics14010080

AMA Style

Veitch S, Radia DH. Recent Advances in the Therapeutic Management of Advanced Systemic Mastocytosis. Diagnostics. 2024; 14(1):80. https://doi.org/10.3390/diagnostics14010080

Chicago/Turabian Style

Veitch, Scott, and Deepti H. Radia. 2024. "Recent Advances in the Therapeutic Management of Advanced Systemic Mastocytosis" Diagnostics 14, no. 1: 80. https://doi.org/10.3390/diagnostics14010080

APA Style

Veitch, S., & Radia, D. H. (2024). Recent Advances in the Therapeutic Management of Advanced Systemic Mastocytosis. Diagnostics, 14(1), 80. https://doi.org/10.3390/diagnostics14010080

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